{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T16:57:12Z","timestamp":1785603432386,"version":"3.56.0"},"reference-count":43,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T00:00:00Z","timestamp":1774915200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T00:00:00Z","timestamp":1774915200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100011104","name":"Universitat Aut\u00f2noma de Barcelona","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100011104","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neural Transm"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Huntington\u2019s disease (HD) is characterized by heterogeneous rates of clinical progression, complicating patient monitoring and clinical trial design. Although speech and language alterations are increasingly recognized as part of the HD cognitive phenotype, their value as short-term prognostic biomarkers of clinically meaningful disease progression and neurodegeneration remains unestablished. In this prospective 12-month longitudinal study, we investigated whether objectively quantified spontaneous speech and language measures predict short-term clinically meaningful progression and relate to biomarkers of neurodegeneration in HD. Eighty-six participants (42 manifest HD, 24 premanifest gene carriers, and 20 healthy controls) underwent baseline spontaneous speech assessment, structural MRI, and plasma neurofilament light chain (NfL) quantification. Clinically meaningful worsening was defined using validated minimal clinically important difference thresholds in the composite Unified Huntington\u2019s Disease Rating Scale (cUHDRS). Spontaneous speech and language measures progressively deteriorated across disease stages and were associated with reduced cortico-subcortical gray matter volume in distributed associative and integrative regions. In manifest HD, logistic regression analyses revealed that baseline language integrity independently predicted clinically meaningful worsening at 12 months (OR\u2009=\u20093.840, 95% CI\u2009=\u20091.46\u201313.33; AUC\u2009=\u20090.783). Combining speech-derived measures with plasma NfL improved discrimination accuracy of individuals with accelerated clinical progression (AUC\u2009=\u20090.807). Spontaneous speech represents an early, accessible and sensitive marker of neurodegeneration in HD. The combination of speech and language derived measures and plasma NfL enables accurate identification of individuals at risk of accelerated, clinically meaningful disease progression, supporting their potential utility as short-term prognostic biomarkers for clinical trials enrichment and stratification.<\/jats:p>","DOI":"10.1007\/s00702-026-03143-x","type":"journal-article","created":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T11:16:59Z","timestamp":1774955819000},"page":"1519-1529","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Spontaneous speech and language measures as predictive biomarkers of clinically meaningful disease progression and neurodegeneration in Huntington\u2019s disease"],"prefix":"10.1007","volume":"133","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8883-4947","authenticated-orcid":false,"given":"Arnau","family":"Puig-Davi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-2590-4307","authenticated-orcid":false,"given":"Carla","family":"Franch-Marti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Caler-Gameiro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9835-0484","authenticated-orcid":false,"given":"Jesus","family":"Perez-Perez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4864-5548","authenticated-orcid":false,"given":"Gonzalo","family":"Olmedo-Saura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9316-1772","authenticated-orcid":false,"given":"Jon","family":"Rodriguez-Antiguedad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9842-7656","authenticated-orcid":false,"given":"Anna","family":"V\u00e1zquez-Oliver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Rivas-Asensio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Perez-Carasol","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarita","family":"Rubio-Romera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Ji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2015-5857","authenticated-orcid":false,"given":"I\u00f1igo","family":"Ruiz-Barrio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3260-7876","authenticated-orcid":false,"given":"Lidia","family":"Bojtos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3933-1355","authenticated-orcid":false,"given":"Frederic","family":"Sampedro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3248-704X","authenticated-orcid":false,"given":"Javier","family":"Pagonabarraga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4870-1431","authenticated-orcid":false,"given":"Jaime","family":"Kulisevsky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0125-7249","authenticated-orcid":false,"given":"Saul","family":"Martinez-Horta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,31]]},"reference":[{"key":"3143_CR1","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/j.cortex.2017.07.017","volume":"95","author":"V Baake","year":"2017","unstructured":"Baake V, Reijntjes RHAM, Dumas EM et al (2017) Cognitive decline in Huntington\u2019s disease expansion gene carriers. Cortex J Devoted Study Nerv Syst Behav 95:51\u201362. https:\/\/doi.org\/10.1016\/j.cortex.2017.07.017","journal-title":"Cortex J Devoted Study Nerv Syst Behav"},{"key":"3143_CR2","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1007\/s11065-021-09481-9","volume":"31","author":"DA Copland","year":"2021","unstructured":"Copland DA, Brownsett S, Iyer K, Angwin AJ (2021) Corticostriatal Regulation of Language Functions. Neuropsychol Rev 31:472\u2013494. https:\/\/doi.org\/10.1007\/s11065-021-09481-9","journal-title":"Neuropsychol Rev"},{"key":"3143_CR3","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1038\/s41582-025-01159-7","volume":"22","author":"J Donaldson","year":"2026","unstructured":"Donaldson J, Hensman Moss D, Ciosi M et al (2026) Huntington disease: somatic expansion, pathobiology and therapeutics. Nat Rev Neurol 22:5\u201321. https:\/\/doi.org\/10.1038\/s41582-025-01159-7","journal-title":"Nat Rev Neurol"},{"key":"3143_CR4","doi-asserted-by":"publisher","first-page":"2858","DOI":"10.1093\/brain\/awm217","volume":"130","author":"A Feigin","year":"2007","unstructured":"Feigin A, Tang C, Ma Y et al (2007) Thalamic metabolism and symptom onset in preclinical Huntington\u2019s disease. Brain J Neurol 130:2858\u20132867. https:\/\/doi.org\/10.1093\/brain\/awm217","journal-title":"Brain J Neurol"},{"key":"3143_CR5","doi-asserted-by":"publisher","first-page":"111048","DOI":"10.1016\/j.pscychresns.2020.111048","volume":"298","author":"LS Furlong","year":"2020","unstructured":"Furlong LS, Jakabek D, Power BD et al (2020) Morphometric in vivo evidence of thalamic atrophy correlated with cognitive and motor dysfunction in Huntington\u2019s disease: The IMAGE-HD study. Psychiatry Res Neuroimaging 298:111048. https:\/\/doi.org\/10.1016\/j.pscychresns.2020.111048","journal-title":"Psychiatry Res Neuroimaging"},{"key":"3143_CR6","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1097\/WNN.0000000000000171","volume":"31","author":"M Gagnon","year":"2018","unstructured":"Gagnon M, Barrette J, Macoir J (2018) Language disorders in Huntington disease: a systematic literature review. Cogn Behav Neurol Off J Soc Behav Cogn Neurol 31:179\u2013192. https:\/\/doi.org\/10.1097\/WNN.0000000000000171","journal-title":"Cogn Behav Neurol Off J Soc Behav Cogn Neurol"},{"key":"3143_CR7","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/j.cortex.2018.08.031","volume":"109","author":"M Giavazzi","year":"2018","unstructured":"Giavazzi M, Daland R, Palminteri S et al (2018) The role of the striatum in linguistic selection: evidence from Huntington\u2019s disease and computational modeling. Cortex J Devoted Study Nerv Syst Behav 109:189\u2013204. https:\/\/doi.org\/10.1016\/j.cortex.2018.08.031","journal-title":"Cortex J Devoted Study Nerv Syst Behav"},{"key":"3143_CR8","volume-title":"Boston Diagnostic Aphasia Examination\u2014Third Edition (BDAE-3)","author":"H Goodglass","year":"2001","unstructured":"Goodglass H, Kaplan E, Barresi B (2001) Boston Diagnostic Aphasia Examination\u2014Third Edition (BDAE-3). PRO-ED, Austin, TX"},{"key":"3143_CR9","doi-asserted-by":"publisher","first-page":"1036","DOI":"10.1002\/mds.29394","volume":"38","author":"JL Hamilton","year":"2023","unstructured":"Hamilton JL, Mills JA, Stebbins GT et al (2023) Defining clinical meaningfulness in Huntington\u2019s disease. Mov Disord Off J Mov Disord Soc 38:1036\u20131043. https:\/\/doi.org\/10.1002\/mds.29394","journal-title":"Mov Disord Off J Mov Disord Soc"},{"key":"3143_CR10","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/j.cortex.2017.07.022","volume":"100","author":"W Hinzen","year":"2018","unstructured":"Hinzen W, Rossell\u00f3 J, Morey C et al (2018) A systematic linguistic profile of spontaneous narrative speech in pre-symptomatic and early stage Huntington\u2019s disease. Cortex J Devoted Study Nerv Syst Behav 100:71\u201383. https:\/\/doi.org\/10.1016\/j.cortex.2017.07.022","journal-title":"Cortex J Devoted Study Nerv Syst Behav"},{"key":"3143_CR11","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1002\/mds.870110204","volume":"11","author":"Huntington Study Group","year":"1996","unstructured":"Huntington Study Group (1996) Unified Huntington\u2019s Disease Rating Scale: reliability and consistency. Huntington Study Group. Mov Disord Off J Mov Disord Soc 11:136\u2013142. https:\/\/doi.org\/10.1002\/mds.870110204","journal-title":"Mov Disord Off J Mov Disord Soc"},{"key":"3143_CR12","doi-asserted-by":"publisher","unstructured":"Jacquemot C, Bachoud-L\u00e9vi A-C (2021) Striatum and language processing: where do we stand? Cognition 213:104785. https:\/\/doi.org\/10.1016\/j.cognition.2021.104785","DOI":"10.1016\/j.cognition.2021.104785"},{"key":"3143_CR13","doi-asserted-by":"publisher","first-page":"807","DOI":"10.1016\/j.biopsych.2020.11.009","volume":"89","author":"EB Johnson","year":"2021","unstructured":"Johnson EB, Ziegler G, Penny W et al (2021) Dynamics of cortical degeneration over a decade in Huntington\u2019s disease. Biol Psychiatry 89:807\u2013816. https:\/\/doi.org\/10.1016\/j.biopsych.2020.11.009","journal-title":"Biol Psychiatry"},{"key":"3143_CR14","doi-asserted-by":"publisher","first-page":"1262","DOI":"10.1111\/ene.15726","volume":"30","author":"T Kouba","year":"2023","unstructured":"Kouba T, Frank W, Tykalova T et al (2023) Speech biomarkers in Huntington\u2019s disease: a cross-sectional study in pre-symptomatic, prodromal and early manifest stages. Eur J Neurol 30:1262\u20131271. https:\/\/doi.org\/10.1111\/ene.15726","journal-title":"Eur J Neurol"},{"key":"3143_CR15","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1007\/s00429-014-0803-z","volume":"220","author":"SC Krall","year":"2015","unstructured":"Krall SC, Rottschy C, Oberwelland E et al (2015) The role of the right temporoparietal junction in attention and social interaction as revealed by ALE meta-analysis. Brain Struct Funct 220:587\u2013604. https:\/\/doi.org\/10.1007\/s00429-014-0803-z","journal-title":"Brain Struct Funct"},{"key":"3143_CR16","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1017\/S1355617716000321","volume":"22","author":"I Labuschagne","year":"2016","unstructured":"Labuschagne I, Cassidy AM, Scahill RI et al (2016) Visuospatial processing deficits linked to posterior brain regions in premanifest and early stage Huntington\u2019s disease. J Int Neuropsychol Soc JINS 22:595\u2013608. https:\/\/doi.org\/10.1017\/S1355617716000321","journal-title":"J Int Neuropsychol Soc JINS"},{"key":"3143_CR17","doi-asserted-by":"publisher","first-page":"212","DOI":"10.1002\/mdc3.12388","volume":"4","author":"GB Landwehrmeyer","year":"2017","unstructured":"Landwehrmeyer GB, Fitzer-Attas CJ, Giuliano JD et al (2017) Data analytics from Enroll-HD, a Global Clinical Research Platform for Huntington\u2019s Disease. Mov Disord Clin Pract 4:212\u2013224. https:\/\/doi.org\/10.1002\/mdc3.12388","journal-title":"Mov Disord Clin Pract"},{"key":"3143_CR18","doi-asserted-by":"publisher","first-page":"1307","DOI":"10.1002\/mds.29430","volume":"38","author":"X-Y Li","year":"2023","unstructured":"Li X-Y, Bao Y-F, Xie J-J et al (2023) Application value of serum neurofilament light protein for disease staging in Huntington\u2019s disease. Mov Disord Off J Mov Disord Soc 38:1307\u20131315. https:\/\/doi.org\/10.1002\/mds.29430","journal-title":"Mov Disord Off J Mov Disord Soc"},{"key":"3143_CR19","doi-asserted-by":"publisher","first-page":"1527","DOI":"10.1007\/s00415-020-09730-6","volume":"267","author":"S Martinez-Horta","year":"2020","unstructured":"Martinez-Horta S, Horta-Barba A, Perez-Perez J et al (2020a) Utility of the Parkinson\u2019s disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington\u2019s disease. J Neurol 267:1527\u20131535. https:\/\/doi.org\/10.1007\/s00415-020-09730-6","journal-title":"J Neurol"},{"key":"3143_CR22","doi-asserted-by":"publisher","first-page":"102415","DOI":"10.1016\/j.nicl.2020.102415","volume":"28","author":"S Martinez-Horta","year":"2020","unstructured":"Martinez-Horta S, Sampedro F, Horta-Barba A et al (2020b) Structural brain correlates of dementia in Huntington\u2019s disease. NeuroImage Clin 28:102415. https:\/\/doi.org\/10.1016\/j.nicl.2020.102415","journal-title":"NeuroImage Clin"},{"key":"3143_CR20","doi-asserted-by":"publisher","first-page":"1871","DOI":"10.1111\/ene.15806","volume":"30","author":"S Martinez-Horta","year":"2023","unstructured":"Martinez-Horta S, Perez-Perez J, Oltra-Cucarella J et al (2023) Divergent cognitive trajectories in early stage Huntington\u2019s disease: A 3-year longitudinal study. Eur J Neurol 30:1871\u20131879. https:\/\/doi.org\/10.1111\/ene.15806","journal-title":"Eur J Neurol"},{"key":"3143_CR21","doi-asserted-by":"publisher","first-page":"1160","DOI":"10.1002\/acn3.52031","volume":"11","author":"S Martinez-Horta","year":"2024","unstructured":"Martinez-Horta S, Perez-Perez J, Perez-Gonzalez R et al (2024) Cognitive phenotype and neurodegeneration associated with Tau in Huntington\u2019s disease. Ann Clin Transl Neurol 11:1160\u20131171. https:\/\/doi.org\/10.1002\/acn3.52031","journal-title":"Ann Clin Transl Neurol"},{"key":"3143_CR23","doi-asserted-by":"publisher","first-page":"36","DOI":"10.3390\/jpm12010036","volume":"12","author":"S Migliore","year":"2022","unstructured":"Migliore S, D\u2019Aurizio G, Scaricamazza E et al (2022) Cognitive reserve in early manifest huntington disease patients: leisure time is associated with lower cognitive and functional impairment. J Pers Med 12:36. https:\/\/doi.org\/10.3390\/jpm12010036","journal-title":"J Pers Med"},{"key":"3143_CR24","doi-asserted-by":"publisher","first-page":"998","DOI":"10.1002\/mds.22007","volume":"23","author":"J Pagonabarraga","year":"2008","unstructured":"Pagonabarraga J, Kulisevsky J, Llebaria G et al (2008) Parkinson\u2019s disease-cognitive rating scale: a new cognitive scale specific for Parkinson\u2019s disease. Mov Disord Off J Mov Disord Soc 23:998\u20131005. https:\/\/doi.org\/10.1002\/mds.22007","journal-title":"Mov Disord Off J Mov Disord Soc"},{"key":"3143_CR25","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1016\/j.parkreldis.2021.04.017","volume":"87","author":"GM Parkin","year":"2021","unstructured":"Parkin GM, Corey-Bloom J, Snell C et al (2021) Plasma neurofilament light in Huntington\u2019s disease: a marker for disease onset, but not symptom progression. Parkinsonism Relat Disord 87:32\u201338. https:\/\/doi.org\/10.1016\/j.parkreldis.2021.04.017","journal-title":"Parkinsonism Relat Disord"},{"key":"3143_CR26","doi-asserted-by":"publisher","first-page":"105173","DOI":"10.1016\/j.ebiom.2024.105173","volume":"104","author":"GM Parkin","year":"2024","unstructured":"Parkin GM, Thomas EA, Corey-Bloom J (2024) Mapping neurodegeneration across the Huntington\u2019s disease spectrum: a five-year longitudinal analysis of plasma neurofilament light. EBioMedicine 104:105173. https:\/\/doi.org\/10.1016\/j.ebiom.2024.105173","journal-title":"EBioMedicine"},{"key":"3143_CR27","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1016\/B978-0-12-801893-4.00006-7","volume":"144","author":"JS Paulsen","year":"2017","unstructured":"Paulsen JS, Miller AC, Hayes T, Shaw E (2017) Cognitive and behavioral changes in Huntington disease before diagnosis. Handb Clin Neurol 144:69\u201391. https:\/\/doi.org\/10.1016\/B978-0-12-801893-4.00006-7","journal-title":"Handb Clin Neurol"},{"issue":"Pt 6","key":"3143_CR28","doi-asserted-by":"publisher","first-page":"1475","DOI":"10.1093\/brain\/111.6.1475","volume":"111","author":"K Podoll","year":"1988","unstructured":"Podoll K, Caspary P, Lange HW, Noth J (1988) Language functions in Huntington\u2019s disease. Brain J Neurol 111(Pt 6):1475\u20131503. https:\/\/doi.org\/10.1093\/brain\/111.6.1475","journal-title":"Brain J Neurol"},{"key":"3143_CR29","doi-asserted-by":"publisher","first-page":"1201","DOI":"10.1002\/acn3.70022","volume":"12","author":"A Puig-Davi","year":"2025","unstructured":"Puig-Davi A, Franch-Marti C, Ruiz-Barrio I et al (2025) Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington\u2019s Disease. Ann Clin Transl Neurol 12:1201\u20131213. https:\/\/doi.org\/10.1002\/acn3.70022","journal-title":"Ann Clin Transl Neurol"},{"key":"3143_CR30","doi-asserted-by":"publisher","unstructured":"Roiboit CM, Stout JC (2025) Cognitive endpoints in Huntington\u2019s disease trials: a conceptual framework for clinical meaningfulness. Mov Disord Clin Pract https:\/\/doi.org\/10.1002\/mdc3.70298","DOI":"10.1002\/mdc3.70298"},{"key":"3143_CR31","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","volume":"10","author":"CA Ross","year":"2011","unstructured":"Ross CA, Tabrizi SJ (2011) Huntington\u2019s disease: from molecular pathogenesis to clinical treatment. Lancet Neurol 10:83\u201398. https:\/\/doi.org\/10.1016\/S1474-4422(10)70245-3","journal-title":"Lancet Neurol"},{"key":"3143_CR32","doi-asserted-by":"publisher","first-page":"369","DOI":"10.3233\/JHD-240021","volume":"13","author":"C Saft","year":"2024","unstructured":"Saft C, Jessen J, Hoffmann R et al (2024) Speech biomarkers in Huntington\u2019s disease: a longitudinal follow-up study in premanifest mutation carriers. J Huntingt Dis 13:369\u2013373. https:\/\/doi.org\/10.3233\/JHD-240021","journal-title":"J Huntingt Dis"},{"key":"3143_CR33","doi-asserted-by":"publisher","first-page":"2625","DOI":"10.1016\/j.neuropsychologia.2012.07.014","volume":"50","author":"S Sambin","year":"2012","unstructured":"Sambin S, Teichmann M, de Diego Balaguer R et al (2012) The role of the striatum in sentence processing: disentangling syntax from working memory in Huntington\u2019s disease. Neuropsychologia 50:2625\u20132635. https:\/\/doi.org\/10.1016\/j.neuropsychologia.2012.07.014","journal-title":"Neuropsychologia"},{"key":"3143_CR34","doi-asserted-by":"publisher","first-page":"2495","DOI":"10.1212\/WNL.0000000000004743","volume":"89","author":"SA Schobel","year":"2017","unstructured":"Schobel SA, Palermo G, Auinger P et al (2017) Motor, cognitive, and functional declines contribute to a single progressive factor in early HD. Neurology 89:2495\u20132502. https:\/\/doi.org\/10.1212\/WNL.0000000000004743","journal-title":"Neurology"},{"key":"3143_CR35","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1212\/wnl.29.1.1","volume":"29","author":"I Shoulson","year":"1979","unstructured":"Shoulson I, Fahn S (1979) Huntington disease: clinical care and evaluation. Neurology 29:1\u20133. https:\/\/doi.org\/10.1212\/wnl.29.1.1","journal-title":"Neurology"},{"key":"3143_CR36","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1016\/S1474-4422(13)70088-7","volume":"12","author":"SJ Tabrizi","year":"2013","unstructured":"Tabrizi SJ, Scahill RI, Owen G et al (2013) Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington\u2019s disease in the TRACK-HD study: analysis of 36-month observational data. Lancet Neurol 12:637\u2013649. https:\/\/doi.org\/10.1016\/S1474-4422(13)70088-7","journal-title":"Lancet Neurol"},{"key":"3143_CR37","doi-asserted-by":"publisher","first-page":"632","DOI":"10.1016\/S1474-4422(22)00120-X","volume":"21","author":"SJ Tabrizi","year":"2022","unstructured":"Tabrizi SJ, Schobel S, Gantman EC et al (2022) A biological classification of Huntington\u2019s disease: the Integrated Staging System. Lancet Neurol 21:632\u2013644. https:\/\/doi.org\/10.1016\/S1474-4422(22)00120-X","journal-title":"Lancet Neurol"},{"key":"3143_CR40","doi-asserted-by":"publisher","first-page":"1155","DOI":"10.1093\/brain\/awh472","volume":"128","author":"M Teichmann","year":"2005","unstructured":"Teichmann M, Dupoux E, Kouider S et al (2005) The role of the striatum in rule application: the model of Huntington\u2019s disease at early stage. Brain J Neurol 128:1155\u20131167. https:\/\/doi.org\/10.1093\/brain\/awh472","journal-title":"Brain J Neurol"},{"key":"3143_CR41","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1162\/jocn.2006.18.9.1555","volume":"18","author":"M Teichmann","year":"2006","unstructured":"Teichmann M, Dupoux E, Kouider S, Bachoud-L\u00e9vi A-C (2006) The role of the striatum in processing language rules: evidence from word perception in Huntington\u2019s disease. J Cogn Neurosci 18:1555\u20131569. https:\/\/doi.org\/10.1162\/jocn.2006.18.9.1555","journal-title":"J Cogn Neurosci"},{"key":"3143_CR39","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1016\/j.neuropsychologia.2007.07.022","volume":"46","author":"M Teichmann","year":"2008","unstructured":"Teichmann M, Dupoux E, Cesaro P, Bachoud-L\u00e9vi A-C (2008) The role of the striatum in sentence processing: evidence from a priming study in early stages of Huntington\u2019s disease. Neuropsychologia 46:174\u2013185. https:\/\/doi.org\/10.1016\/j.neuropsychologia.2007.07.022","journal-title":"Neuropsychologia"},{"key":"3143_CR38","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1016\/j.cortex.2008.12.005","volume":"45","author":"M Teichmann","year":"2009","unstructured":"Teichmann M, Darcy I, Bachoud-L\u00e9vi A-C, Dupoux E (2009) The role of the striatum in phonological processing: evidence from early stages of Huntington\u2019s disease. Cortex J Devoted Study Nerv Syst Behav 45:839\u2013849. https:\/\/doi.org\/10.1016\/j.cortex.2008.12.005","journal-title":"Cortex J Devoted Study Nerv Syst Behav"},{"key":"3143_CR42","doi-asserted-by":"publisher","first-page":"e617","DOI":"10.1212\/NXG.0000000000000617","volume":"7","author":"PA Wijeratne","year":"2021","unstructured":"Wijeratne PA, Garbarino S, Gregory S et al (2021) Revealing the timeline of structural MRI changes in premanifest to manifest Huntington disease. Neurol Genet 7:e617. https:\/\/doi.org\/10.1212\/NXG.0000000000000617","journal-title":"Neurol Genet"},{"key":"3143_CR43","doi-asserted-by":"publisher","first-page":"751","DOI":"10.1002\/ajmg.b.31232","volume":"156B","author":"Y Zhang","year":"2011","unstructured":"Zhang Y, Long JD, Mills JA et al (2011) Indexing disease progression at study entry with individuals at-risk for Huntington disease. Am J Med Genet Part B Neuropsychiatr Genet Off Publ Int Soc Psychiatr Genet 156B:751\u2013763. https:\/\/doi.org\/10.1002\/ajmg.b.31232","journal-title":"Am J Med Genet Part B Neuropsychiatr Genet Off Publ Int Soc Psychiatr Genet"}],"container-title":["Journal of Neural Transmission"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00702-026-03143-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00702-026-03143-x","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00702-026-03143-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T16:07:05Z","timestamp":1785600425000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00702-026-03143-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,31]]},"references-count":43,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["3143"],"URL":"https:\/\/doi.org\/10.1007\/s00702-026-03143-x","relation":{},"ISSN":["0300-9564","1435-1463"],"issn-type":[{"value":"0300-9564","type":"print"},{"value":"1435-1463","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,31]]},"assertion":[{"value":"2 February 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"31 March 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Informed consent was obtained from all participants prior to their inclusion in the study. All procedures were conducted in accordance with the 1964 Helsinki Declaration and its later amendments and approved by ethics committee from the Hospital de la Santa Creu i Sant Pau (approval code IIBSP-FPI-2021-128).","order":2,"name":"Ethics","label":"Ethical approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Informed consent was obtained from all participants prior to study inclusion. All procedures were conducted in accordance with the 1964 Helsinki Declaration and its later amendments and approved by ethics committee from the Hospital de la Santa Creu i Sant Pau (approval code IIBSP-FPI-2021-128).","order":3,"name":"Ethics","label":"Informed consent","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Written informed consent was obtained from all individual participants included in the study.","order":4,"name":"Ethics","label":"Consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}}]}}